Natural history of idiopathic erythrocytosis - A retrospective case series review

Idiopathic Erythrocytosis (IE) is a diagnosis given to patients who have an absolute erythrocytosis (red cell mass more than 25% above their mean normal predicted value) but who do not have a known form of primary or secondary erythrocytosis (BCSH guideline, 2005). We report here the results of a follow-up study of 80 patients (44 male and 36 female) diagnosed with IE from the United Kingdom and the Republic of Ireland over a 10 year period. Baseline information was initially collected when investigating for molecular causes of erythrocytosis in this group. The diagnosis of IE was made on the basis of a raised red cell mass >25% above mean normal predicted value, absence of Polycythaemia Vera (PV) based on the criteria of Pearson and Messinezy (1996), and the exclusion of secondary erythrocytosis (oxygen saturation >92% on pulse oximetry, no history of sleep apnoea, no renal or hepatic pathology, and a normal oxygen dissociation curve (if indicated). The average age at diagnosis of erythrocytosis was 34.5 (2–74 years). Erythropoietin levels were available for 77/80 of the patients and were low in 18 (23%) and normal or high in 59 (74%). Ultrasound imaging was carried out in 67 patients (84%) at time of diagnosis and no significant abnormalities found. Fourteen patients had a family history of erythrocytosis. These patients have now been followed up for an average of 9.4 years (range 1–39). Out of 80 patients 56 patients can still be classified as having IE, of whom 52 are living (cause of death in the other 4 - lung cancer, RTA, sepsis, unknown). Thirty-five of these patients are regularly venesected, 3 take hydroxyurea (one also venesected), 11 receive no treatment while treatment is unknown in 2. Twenty take aspirin, 1 warfarin and 31 no thromboprophylaxis. Four of these patients had suffered thromboembolic complications (3 with CVA/TIAs and 1 with recurrent DVT) at or before their original diagnosis. Since diagnosis 8 patients have had 9 thrombotic events of which 7 were arterial (1 CVA, 3 TIAs, 1 MI, 2 PVD) and 2 venous (DVT/PE). Twenty take aspirin, 1 dipyridamole, 1 warfarin and 30 take no thromboprophylaxis. Out of the 24 patients who now have a diagnosis other than IE, 8 have been diagnosed with myelo-proliferative disease. Thirteen patients have a molecular abnormality which is likely to account for their erythrocytosis (11 VHL, 1 PHD-2, 1 EPO-receptor mutations). Three patients have secondary erythrocytosis. Older case studies identified a heterogenous group of patients, some of whom probably had apparent erythrocytosis and some who had either primary polycythaemia or secondary causes later identified (Modan and Modan, Najean et al). More recent reviews have identified a more homogenous group with low rates of transformation to myelofibrosis/acute leukaemia and low rates of thrombosis of around 1% patient-year. Follow up of our initial patient group does indeed reveal a heterogeneous group of patients with 10% now diagnosed with an MPD, although when analysis is confined to those patients who continue to fulfil the criteria for IE, the clinical course has been more stable. There has been no progression to MDS or leukaemia in this group (one patient with PV progressed to AML). The rate of thrombosis is 1.6% patient-years which is lower than the rate seen in PV and is consistent with the rate identified in other series. Molecular defects continue to be identified in this group and future investigation is likely to reveal further abnormalities.

Discovery and evolving history of two genetically related but phenotypically different viruses, porcine circoviruses 1 and 2

Porcine circoviruses (PCVs) belong to the genus Circovirus, family Circoviridae. and are the smallest non-enveloped, single stranded, negative sense, circular DNA viruses that replicate autonomously in mammalian cells. Two types of PCV have been characterised, PCV1 and PCV2 and these two viruses show 83% sequence identity at open reading frame (ORF) 1 and 67% identity at ORF2. PCV1 is a nonpathogenic virus of pigs. In contrast, PCV2 has emerged as a major pathogen of swine around the world. The discovery of PCV1 and how the subsequent studies on this virus eventually led to the recognition and characterisation of PCV2, and the disease scenarios associated with PCV2, serve as a model of how multidisciplinary collaboration among field veterinarians, diagnosticians and researchers can lead to the rapid characterisation and control of a globally important emerging disease. (C) 2011 Elsevier B.V. All rights reserved.

Postglacial vegetation dynamics of western Tierra del Fuego

The southern fringes of the South American landmass provide a rare opportunity to examine the development of moorland vegetation with sparse tree cover in a wet, cool temperate climate of the Southern Hemisphere. We present a record of changes in vegetation over the past 17,000 years, from a lake in extreme southern Chile (Isla Santa Inés, Magallanes region, 53°38.97S; 72°25.24W), where human influence on vegetation is negligible. The western archipelago of Tierra del Fuego remained treeless for most of the Lateglacial period; Lycopodium magellanicum, Gunnera magellanica and heath species dominated the vegetation. Nothofagus may have survived the last glacial maximum at the eastern edge of the Magellan glaciers from where it spread southwestwards and established in the region at around 10,500 cal. yr BP. Nothofagus antarctica was likely the earlier colonizing tree in the western islands, followed shortly after by Nothofagus betuloides. At 9000 cal. yr BP moorland communities expanded at the expense of Nothofagus woodland. Simultaneously, Nothofagus species shifted to dominance of the evergreen Nothofagus betuloides and the Magellanic rain forest established in the region. Rapid and drastic vegetation changes occurred at 5200 cal. yr BP, after the Mt Burney MB2 eruption, including the expansion and establishment of Pilgerodendron uviferum and the development of mixed Nothofagus-Pilgerodendron-Drimys woodland. Scattered populations of Nothofagus, as they occur today in westernmost Tierra del Fuego may be a good analogue for Nothofagus populations during the Lateglacial in eastern sites.

Experimental and Numerical Evaluation of Thermal Performance of Steered Fibre Composite Laminates

For Variable Stiffness (VS) composites with steered curvilinear tow paths, the fiber orientation angle varies continuously throughout the laminate, and is not required to be straight, parallel and uniform within each ply as in conventional composite laminates. Hence, the thermal properties (conduction), as well as the structural stiffness and strength, vary as functions of location in the laminate, and the associated composite structure is often called a “variable stiffness” composite structure. The steered fibers lead not only to the alteration of mechanical load paths, but also to the alteration of thermal paths that may
result in favorable temperature distributions within the laminate and improve the laminate performance. Evaluation of VS laminate performance under thermal loading is the focus of this chapter. Thermal performance evaluations require experimental and numerical analysis of VS laminates under different processing and loading conditions. One of the advantages of using composite materials in many applications is the tailoring capability of the laminate,
not only during the design phase but also for manufacturing. Heat transfer through variable conduction and chemical reaction (degree of cure) occurring during manufacturing (curing) plays an important role in the final thermal and mechanical performance, and shape of composite structures.

Reliability Assessment of 3-D Space Frame Structures Applying Stochastic Finite Element Analysis

Throughout design development of satellite structure, stress engineer is usually challenged with randomness in applied loads and material properties. To overcome such problem, a risk-based design is applied which estimates satellite structure probability of failure under static and thermal loads. Determining probability of failure can help to update initially applied factors of safety that were used during structure preliminary design phase. These factors of safety are related to the satellite mission objective. Sensitivity-based analysis is to be implemented in the context of finite element analysis (probabilistic finite element method or stochastic finite element method (SFEM)) to determine the probability of failure for satellite structure or one of its components.

Improvement of the Performance of Hysteresis Compensation in SMA Actuators by Using Inverse Preisach Model in Closed – Loop Control System

The aim of this paper is to increase the performance of hysteresis compensation for Shape Memory Alloy (SMA) actuators by using inverse Preisach model in closed — loop control system. This is used to reduce hysteresis effects and improve accuracy for the displacement of SMA actuators. Firstly, hysteresis is identified by numerical Preisach model implementation. The geometrical interpretation from first order transition curves is used for hysteresis modeling. Secondly, the inverse Preisach model is formulated and incorporated in closed-loop PID control system in order to obtain desired current-to-displacement relationship with hysteresis reducing. The experimental results for hysteresis compensation by using this method are also shown in this paper.